Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome are acute, fulminant diseases characterized by the development of thrombocytopenia and microangiopathic hemolytic anemia.
Idiopathic (immune) thrombocytopenic purpura is a hemorrhagic disease caused by thrombocytopenia, not associated with a systemic disease. It usually has a chronic course in adults, but is often acute and transient in children. Spleen size is normal.
Hereditary intracellular disturbances of platelets are rare diseases and lead to the manifestation of bleeding throughout life. The diagnosis is confirmed by the study of platelet aggregation. In the presence of severe manifestations of bleeding, transfusion of platelets is necessary.
Platelets are fragments of megakaryocytes that provide hemostasis of circulating blood. Thrombopoietin is synthesized by the liver in response to a decrease in the number of bone marrow megakaryocytes and circulating platelets and stimulates the bone marrow to the synthesis of platelets from megakaryocytes.
The consequences of lymphopenia include the development of opportunistic infections and an increased risk of cancer and autoimmune diseases. If lymphopenia is detected when performing a general blood test, diagnostic tests should be performed for immunodeficient states and analysis of lymphocyte subpopulations. The treatment is aimed at the underlying disease.
Neutropenia (agranulocytosis, granulocytopenia) is a decrease in the number of blood neutrophils (granulocytes). In severe neutropenia, the risk and severity of bacterial and fungal infections increase.
Because of the high incidence of thalassemia HbS and beta-thalassemia in certain population groups, the congenital presence of both anomalies is quite frequent.
Symptoms and complaints are caused by the development of anemia, hemolysis, splenomegaly, bone marrow hyperplasia and multiple transfusions with iron overload. The diagnosis is based on a quantitative analysis of hemoglobin.
Hemoglobin E is the third most frequent occurrence of hemoglobinopathy (after HbA and HbS), it occurs mainly in Negroid and American people and people from Southeast Asia, while among Chinese it is rare.
